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Die Ausscheidung von β-Aminoisobuttersäure bei Gesunden und Kranken

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Zusammenfassung

1. Bei größerer interindividueller Schwankung zeigt die Ausscheidung von β-AIB im Urin eine erstaunliche intraindividuelle Konstanz.

2. Untersuchungen von Leberkranken ergaben, daß der Funktionszustand der Leber keinen wesentlichen Einfluß auf die Ausscheidung von β-AIB im Urin hat.

3. Die Hälfte der untersuchten Patienten mit Hämochromatose scheidet größere Mengen dieser Aminosäure aus.

4. Der bei der Ahorn-Sirup-Krankheit auftretende Enzymdefekt (Ausfall der α-Ketosäureoxydasen) hat keinen Einfluß auf die Menge der eliminierten β-AIB.

Summary

1. β-AIB excretion varies greatly from individual to individual, whereas normal day to day differences in β-AIB excretion rates for any one person appear to remain constant.

2. Impaired liver function does not markedly alter urinary β-AIB excretion.

3. Half of our patients with hemochromatosis excrete large amounts of β-AIB.

4. Impaired activities of α-ketoacid-oxydases in maple syrup urine disease have no apparent effect on β-AIB excretion.

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Abbreviations

β-AIB:

β-Aminoisobuttersäure

DNFB:

2,4 Dinitro-1-Fluorobenzol

Literatur

  1. Armstrong, M. D., K. Yates, Y. Kakimoto, K. Taniguchi, andTh. Kappe: Excretion of β-aminoisobutyric acid by man. J. biol. Chem.238, 1447 (1963).

    Google Scholar 

  2. Awapara, J., andC. C. Shullenberger: Urinary excretion of β-aminoisobutyric acid after administration of thymine and nitrogen mustard. Clin. chim. Acta2, 199 (1957).

    Google Scholar 

  3. Calchi-Novati, C., R. Ceppellini, I. Blancho, E. Silvestroni, andH. Harris: β-aminoisobutyric acid excretion in urine. Ann. Eugen. (Lond.)18, 335 (1954).

    Google Scholar 

  4. Crumpler, H. R., C. E. Dent, H. Harris, andR. G. Westall: β-Aminoisobutyric acid (a methyl-β-alanine): a new amino acid obtained from human urine. Nature (Lond.)167, 307 (1951).

    Google Scholar 

  5. Dent, C. E., andJ. M. Walshe: Amino acid metabolism in liver disease. In: Liver Disease (Ciba Foundation Symposium) (S. Sherlock, andG. E. W. Wolstenholme, Ed.), p. 22–30. New York: McGraw-Hill-Blakiston 1951.

    Google Scholar 

  6. Edozier, J. C., E. J. Phillips, andW. R. F. Collis: The free amino acids of plasma and urine in kwashiorkor. Lancet1960 I, 615.

    Google Scholar 

  7. Evered, D. F.: The excretion of amino acids by the human. Biochem. J.62, 416 (1956).

    Google Scholar 

  8. Fink, K., R. B. Henderson, andR. M. Fink: β-aminoisobutyric acid, a possible factor in pyrimidine metabolism. Proc. Soc. exp. Biol. (N. Y.)78, 135 (1951).

    Google Scholar 

  9. Fink, K., R. E. Cline, R. B. Henderson, andR. M. Fink: Metabolism of thymine (methyl-C14 or -2-C14) by rat liver in vitro. J. biol. Chem.221, 425 (1956).

    Google Scholar 

  10. Gartler, S. M.: A family study of urinary β-aminoisobutyric acid excretion. Amer. J. hum. Genet.8, 120 (1956).

    Google Scholar 

  11. Gartler, S. M., I. L. Firschein, andT. Gidaspon: Some genetical and anthropological considerations of urinary β-aminoisobutyric acid excretion. Acta genet. (Basel)6, 435 (1957).

    Google Scholar 

  12. Gartler, S. M.: An investigation into the biochemical genetics of β-aminoisobutyric aciduria. Amer. J. hum. Genet.11, 257 (1959).

    Google Scholar 

  13. Geiss, F., H. Schlitt, F. J. Ritter u.W. M. Weimar: Analyse von Polyphenylgemischen mit der Dünnschichtchromatographie. J. Chromatog.12, 469 (1963).

    Google Scholar 

  14. Goedde, H. W., E. Richter, C. Stahlmann u.B. Sixel: Heterozygotentest für die Ahorn-Sirup-Krankheit, Klin. Wschr.41, 953 (1963) und42, 818 (1964) sowie „Humangenetik“ 1964 (im Druck).

    Google Scholar 

  15. Goedde, H. W., andH. Brunschede: β-Aminoisobutyric acid: a thin layer chromatographic method for the quantitative estimation in human urine. Clin. chim. Acta (im Druck).

  16. Grouchy, J. de, andH. E. Sutton: A genetic study of β-aminoisobutyric acid excretion. Amer. J. hum. Genet.9, 76 (1957).

    Google Scholar 

  17. Harris, H.: Family studies on the urinary excretion of β-aminoisobutyric acid. Ann. Eugen. (Lond.)18, 43 (1953).

    Google Scholar 

  18. Kupiecki, F. P., andM. J. Coon: The enzymatic synthesis of β-aminoisobutyrate, a product of valine metabolism, and of β-alanine, a product of β-hydroxypropionate metabolism. J. biol. Chem.229, 743 (1957).

    Google Scholar 

  19. Levey, St., Th. Woods, andW. E. Abbott: Urinary excretion of β-aminoisobutyric acid following surgical procedures. Metabolism12, 148 (1963).

    Google Scholar 

  20. Lundin, L. G., andK. H. Gustavson: Urinary BAIB excretion in Down's syndrome (Mongolism). Acta genet. (Basel)12, 156 (1962).

    Google Scholar 

  21. McEvoy-Bowe, E.: Family studies of urinary β-aminoisobutyric acid excretion together with an examination of the urinary excretion levels of glycine, alanine, taurine, and glutamine amongst Chinese in Singapore. Ann. hum. Genet.25, 331 (1962).

    Google Scholar 

  22. Peraino, C., andA. E. Harper: Quantitative chromatography of plasma amino acids. Analyt. Chem.33, 1863 (1961).

    Google Scholar 

  23. Rubini, J. R., E. P. Cronkite, V. P. Bond, andT. M. Fliedner: Urinary excretion of β-aminoisobutyric acid (BAIB) in irridiated human beings. Proc. Soc. exp. Biol. (N. Y.)100, 130 (1959).

    Google Scholar 

  24. Schendel, H. E., A. Antonis, andJ. D. L. Hansen: Increased amino aciduria in infants with kwashiorkor. Pediatrics23, 662 (1959).

    Google Scholar 

  25. Soupart, P.: Free amino acids of blood and urine in the human. In: Amino Acid Pools (J. T. Holden, Ed.), p. 220 to 262. New York: Elsevier Publ. Co. 1962.

    Google Scholar 

  26. Sutton, H. E.: β-Aminoisobutyricaciduria. In: The Metabolic Bais of Inherited Disease (J. B. Stanbury, J. B. Wyngaarden, andD. S. Fredrickson, Ed.), p. 792. New York: McGraw-Hill Book Co. 1960.

    Google Scholar 

  27. Walz, D., A. R. Fahmy, G. Pataki, A. Niederwieser u.M. Brenner: Dünnschichtchromatographischer Nachweis der Aminosäuren im Urin. Experientia (Basel)19, 213 (1963).

    Google Scholar 

  28. Westall, R. G., J. Dancis, S. Miller, andM. Levitz: Maple sugar urine disease. Fed. Proc.17, 334 (1958).

    Google Scholar 

  29. Wright, S. W., andK. Fink: The excretion of β-aminoisobutyric acid in normal, mongoloid, and non-mongoloid mentally defective children. Amer. J. ment. Defic.61, 530 (1957).

    Google Scholar 

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Authors and Affiliations

  1. Aus der Abteilung für Biochemische Genetik, Anthropologisches Institut der Universität Freiburg i. Br., Germany

    H. Brunschede, R. Hoffbauer & H. W. Goedde

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  1. H. Brunschede
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  2. R. Hoffbauer
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  3. H. W. Goedde
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Brunschede, H., Hoffbauer, R. & Goedde, H.W. Die Ausscheidung von β-Aminoisobuttersäure bei Gesunden und Kranken. Klin Wochenschr 43, 93–97 (1965). https://doi.org/10.1007/BF01485781

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